Functions of the mitochondria and its role in associated pathologies

Authors

  • Alexander Cobos Muñoz Catholic University of Santiago de Guayaquil image/svg+xml
  • Juan Andres Gómez Parra Catholic University of Santiago de Guayaquil image/svg+xml
  • Juan Jose Gómez Parra Universidad Técnica de Babahoyo image/svg+xml
  • Denny Rafael Córdova Cercado Universidad Técnica de Manabí image/svg+xml

Keywords :

Mitochondria, energy production, metabolism, diseases

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Abstract

Mitochondria, an organelle present in eukaryotic cells, play a vital role in energy production and regulation of metabolism. Discovered in 1898, its complex structure comprises highly folded external and internal membranes. The electron transport chain, located in the inner membrane, generates an electrochemical gradient essential for the synthesis of ATP, the main source of cellular energy. In addition to its energetic function, mitochondria participate in the regulation of metabolism, being crucial in the oxidation of fatty acids, synthesis of amino acids and nucleotides, and in apoptosis. Mitochondrial dysfunctions, associated with diseases such as Parkinson's and Alzheimer's, have been the subject of intense research. The study of clinical cases, such as Kearns-Sayre syndrome, highlights the diversity of manifestations and the importance of early diagnosis. The specialized structure of the mitochondria and its connection with pathologies underline its relevance in cellular and molecular biology. Understanding mitochondrial alterations is essential to develop therapeutic strategies and advance personalized medicine, highlighting the continued need for research in this field.

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References

Alberts B, Johnson A, Lewis J, Raff M, Roberts K, Walter P. Molecular biology of the cell. 6th ed. New York: Garland Science; 2014.

Alberts B, Johnson A, Lewis J. Molecular Biology of the Cell. 4th edition. New York: Garland Science; 2002. The Mitochondrion. Available from: https://www.ncbi.nlm.nih.gov/books/NBK26871/.

Aura V. Org.co. [citado el 26 de marzo de 2023]. Disponible en: http://www.scielo.org.co/pdf/anco/v32n3/v32n3a09.pdf.

Becker T, Pfanner N, Wiedemann N. Mitochondrial Outer Membrane: Diversity in Form and Function. J Mol Biol. 2021;433(15):166987. doi: 10.1016/j.jmb.2021.166987.

Blakely, E. L., He, L., Gardner, J. L., Hudson, G., Walter, J. W., Hughes, I., .y Taylor, R. W. Novel mutations in ND3, ATP6 and ATP8 associated with profound OXPHOS defects and Leigh syndrome. Journal of medical genetics. 2013; 50(5), 262-270. doi: 10.1136/jmedgenet-2012-101381.

Cohen, S., Myers, J., & Fisher, P. (2018). Mitochondrial Encephalomyelitis: A Comprehensive Overview. Neurology, 30(4), 512–521.

DiMauro, S., & Schon, E. A. (2013). Mitochondrial disorders in the nervous system. Annual Review of Neuroscience, 36, 125–147.

D'Ortencio A., Navigante A. Disfunción mitocondrial y enfermedades cardiovasculares. Insuf. card. [Internet]. 2016 dic [citado 2023 Mar 22]; 11(4): 201-214. Disponible en: http://www.scielo.org.ar/scielo.php?script=sci_arttext&pid=S1852-38622016000400005&lng=es.

Duque, E. (2015). Seminario Metodología de la Investigación, Universidad Nacional de Colombia.

Galluzzi L, Kepp O, Chan FK-M, Kroemer G. Necroptosis and extracellular matrix damage in stroke with implications for personalized medicine. EMBO Molecular Medicine. 2020; 12(6): e11523.

Galluzzi, L., Vitale, I., Aaronson, S. A., Abrams, J. M., Adam, D., Agostinis, P., … Kroemer, G. (2020). Molecular mechanisms of cell death: recommendations of the Nomenclature Committee on Cell Death 2018. Cell Death & Differentiation, 25(3), 486–541.

Harden A, Young WJ. The alcoholic ferment of yeast-juice. Part II.–The co-enzyme of yeast-juice. Proc R Soc Lond B Biol Sci. 1913; 85(578): 305-339. Available from: https://www.jstor.org/stable/81016.

Jones, A. M., & Williams, C. (2017). Neuroimaging in Kearns-Sayre Syndrome: A Case Series and Literature Review. Journal of Neuroimaging, 27(5), 489–496.

Kerner, J., & Hoppel, C. (2018). Fatty acid import into mitochondria. Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids, 1863(9), 1793-1800. https://doi.org/10.1016/j.bbalip.2018.06.011.

Lane N, Martin WF. The origin of membrane bioenergetics. Cell. 2012;151(7):1406-16. doi: 10.1016/j.cell.2012.11.050. PMID: 23217706.

Lee C, Kim Y, Lee S, Kim Y, Kim J, Lee S. Mitochondria and the Regulation of Energy Homeostasis. Journal of Cellular Physiology. 2022; 237(1): 52-61.

Lee, S. H., Kim, J. S., & Kim, E. Y. (2022). Mitochondrial respiratory chain complex assembly and function. BMB Reports, 55(1), 1-11. https://doi.org/10.5483/BMBRep.2022.55.1.282.

Li, Y., Xu, S., Mihai, D. M., & Zhou, L. (2019). Mitochondrial dysfunction and obesity. Biomolecules, 9(11), 1-17. doi: 10.3390/biom9110701.

Lill, R. (2009). Function and biogenesis of iron–sulphur proteins. Nature, 460(7257), 831–838.

Lodish, H., Berk, A., Zipursky, S. L., Matsudaira, P., Baltimore, D., & Darnell, J. (2000). Molecular cell biology (4th ed.). W. H. Freeman.

Mills EL, Kelly B, O'Neill LAJ. Mitochondria are the powerhouses of immunity. Nat Immunol. 2017;18(5):488-98. doi: 10.1038/ni.3704. PMID: 28436444.

Nicholls DG, Ferguson SJ. Bioenergetics 4. Elsevier; 2013. 13-25 (2), The Structural Organization of the Mitochondrion; Available from: https://www.sciencedirect.com/science/article/pii/B9780123884251000026.

Rambold, A. S., & Pearce, E. L. Mitochondrial dynamics at the interface of immune cell metabolism and function. Trends in Immunology.2021; 42(4), 309-323.

Rocha M, Cardoso SM, Pereira C. Mitochondrial Dysfunction: An Underlying Link in the Pathophysiology of Neurodegenerative Diseases. Pharmaceuticals (Basel). 2021;14(2):127. doi: 10.3390/ph14020127. PMID: 33530544; PMCID: PMC7915747.

Rocha, A. G., Franco, A., Krezel, A. M., Rumsey, J. M., Alberti, J. M., Knight, W. C., . & Baloh, R. H. (2021). MFN2 agonists reverse mitochondrial defects in preclinical models of Charcot-Marie-Tooth disease type 2A. Science, 372(6544), 545-552.

Schon EA, DiMauro S. Mitochondrial disorders. In: Scriver CR, Beaudet AL, Sly WS, Valle D, Childs B, Kinzler KW, Vogelstein B, editors. The metabolic and molecular bases of inherited disease. 8th ed. New York: McGraw-Hill; 2001. p. 2337-78.

Sena LA, Chandel NS. Physiological roles of mitochondrial reactive oxygen species. Molecular Cell. 2012; 48(2): 158-167.

Smith, R. L., Jones, A. M., & Williams, C. (2015). Kearns-Sayre Syndrome: A Comprehensive Review of Clinical Findings, Biochemical Alterations, and Pathogenesis. Neurology Research International, 2015, 1–9.

Suliman HB, Piantadosi CA. Mitochondrial Quality Control as a Therapeutic Target. Pharmacological Reviews. 2016; 68(1): 20-48.

Voet D, Voet JG, Pratt CW. (2016). Fundamentals of Biochemistry: Life at the Molecular Level. 5th edition. Hoboken, NJ: John Wiley & Sons, Inc.; Chapter 18, Mitochondria and Chloroplasts; p. 831-872.

Wallace DC, Chalkia D. Mitochondrial DNA genetics and the heteroplasmy conundrum in evolution and disease. Cold Spring Harb Perspect Biol. 2013 Nov 1;5(11): a021220. doi: 10.1101/cshperspect. a021220. PMID: 24184698; PMCID: PMC3800145.

Wallace DC. Mitochondria and cancer. Nat Rev Cancer. 2012;12(10):685-698. doi:10.1038/nrc3365.

Wallace DC. Mitochondrial DNA mutations in disease and aging. Environmental and molecular mutagenesis. 2010 jun;51(5):440-50.

Wallace, D. C. Mitochondrial DNA mutations in disease and aging. Environmental and Molecular Mutagenesis. 2018; 59(3), 201-202. doi: 10.1002/em.22146.

Warburg O. Über den Stoffwechsel der Carcinomzelle. Biochem Z. 1925; 152: 319–344. Available from: https://www.jstor.org/stable/10.1515/bchm2.1925.152.1-6.319.

West AP, Shadel GS. (2017). Mitochondrial DNA in innate immune responses and inflammatory pathology. Nat Rev Immunol. 2017;17(6):363-75. doi: 10.1038/nri.21. PMID: 28466835.

Published

2024-01-04
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How to Cite

Functions of the mitochondria and its role in associated pathologies. (2024). Journal of Science and Research: Revista Ciencia E Investigación, 9(1), 183-196. https://revistas.utb.edu.ec/index.php/sr/article/view/3057

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Artículo de Revisión